Long-Term Outcomes After the Fontan Operation

Study Questions:

What are the long-term outcomes of patients undergoing the Fontan procedure?


A retrospective review was performed at a single center. Records of all patients undergoing a modified Fontan procedure at the Mayo Clinic between 1973 and 2012 were reviewed. Survival was determined by review of medical records, family contact, and use of the National Death Index. A questionnaire was sent to patients known to be alive at the time of the study.


Of the 1,052 patients included in the study, the underlying cardiac diagnosis was tricuspid atresia in 273 (26%), double inlet left ventricle in 271 (26%), heterotaxy in 135 (13%), pulmonary atresia/intact ventricular septum in 35 (3%), hypoplastic left heart syndrome in 24 (2%), and other in 314 (30%). Overall, 10-, 20-, and 30-year survival was 74%, 61%, and 43%, respectively. Factors associated with decreased overall or late survival in multivariate analysis included preoperative diuretic use, longer cardiopulmonary bypass time, operation prior to 1991, atrioventricular valve replacement at the time of Fontan, elevated post-bypass Fontan (>20 mm Hg) or left atrial (>13 mm Hg) pressures, prolonged chest tube drainage (>21 days), postoperative ventricular arrhythmias, renal insufficiency, and development of protein-losing enteropathy (PLE). For patients surviving at least 30 days after original Fontan operation, the 30-year freedom from death or reoperation was 33%, with most common late reoperations including pacemaker insertion/revision, Fontan revision/conversion, and atrioventricular valve repair or replacement. Clinically significant late atrial or ventricular arrhythmias occurred in 468 patients (44%), and 85 patients (9%) developed PLE, with 5-, 10-, and 20-year survival after diagnosis of PLE was 50%, 35%, and 19%, respectively.


Survival after Fontan has improved over the last 40 years, although development of PLE, arrhythmias, and need for re-operation remain significant challenges.


The Fontan procedure has evolved significantly since its inception. Surgical techniques have changed, with most patients undergoing lateral tunnel or extracardiac Fontan procedures in the more recent surgical era. Additionally, the cardiac substrate has changed, with a larger number of patients with single right ventricles undergoing palliation to the Fontan. Despite these changes, the physiologic basis of the Fontan, with risk of increased systemic venous pressure, remains. The overall long-term survival of 43% is indicative of the challenges of the care of the adult with Fontan circulation. In this cohort, the mortality associated with Fontan conversion was 10%, which contributed to the overall mortality reported in the study. Decisions as to the optimal timing of interventions such as Fontan conversion and transplantation continue to present major challenges to the care of this complex population. Although advances in therapy for PLE may decrease risks for future patients, the development of PLE in this population was ominous, with 19% survival 20 years after a PLE diagnosis.

Clinical Topics: Arrhythmias and Clinical EP, Cardiac Surgery, Congenital Heart Disease and Pediatric Cardiology, Invasive Cardiovascular Angiography and Intervention, Valvular Heart Disease, Implantable Devices, SCD/Ventricular Arrhythmias, Atrial Fibrillation/Supraventricular Arrhythmias, Cardiac Surgery and Arrhythmias, Cardiac Surgery and CHD & Pediatrics, Cardiac Surgery and VHD, Congenital Heart Disease, CHD & Pediatrics and Arrhythmias, CHD & Pediatrics and Interventions, Interventions and Structural Heart Disease

Keywords: Arrhythmias, Cardiac, Cardiac Surgical Procedures, Cardiopulmonary Bypass, Fontan Procedure, Heart Defects, Congenital, Hypoplastic Left Heart Syndrome, Protein-Losing Enteropathies, Pulmonary Atresia, Tricuspid Atresia, Ventricular Septum

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